Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease that, while considered rare, has a prognosis worse than many forms of cancer. It presents with nonspecific symptoms including cough, breathlessness and fatigue. This review provides an overview of the approach to diagnosis and the management options available, including the key role of an interstitial lung disease multidisciplinary team. It also highlights the central co-ordinating role of GPs in IPF patient care.
- Idiopathic pulmonary fibrosis (IPF) is an irreversible, progressive lung disease that has significant mortality.
- A comprehensive multidisciplinary team meeting is key in establishing a diagnosis of IPF.
- New antifibrotic therapies should be considered to slow the progression of IPF in appropriate patients.
- Nonpharmacological measures are essential in the treatment of IPF, and include oxygen, pulmonary rehabilitation, treating comorbidities and referral to palliative care.